Chiari Connection International (CCI) --- The Best In Chiari Resources Worldwide!!

Who do I contact if I have a problem viewing the website or other issues?

If you are having problems viewing the website or have other suggestions for website content or anything else use the "Contact Us" link. Click Here to contact us. The request will be sent to all people involved in the website and the support/information group

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What Does The Support/Information Group Provide?

The Chiari Connection International Support/Information group provides support and information for those who are affected by Arnold Chiari Malformation and related disorders. Related disorders can include: syringomyelia (SM); basilar invagination (BI); cranio-spinal instability (CSI); intracranial and intraspinal cysts; tumors of the cerebellum, brain stem and spinal cord; spina bifida (SB); hydrocephalus; pseudotumor cerebri (PTC); Ehlers Danlos Syndrome (EDS); and various connective tissue disorders.

Some people join because they need support in finding doctors, emotional support, and answers to questions they may have about their condition. Others join simply for information about the disorders. The group is also for family members and friends that want to know more about the condition(s) that are affecting the ones that are important in their lives.

The group is made up of diverse individuals who either have just been diagnosed, are getting ready for procedures, or are looking for answers to problems they are having. Some have been part of similar groups for many years and others have just been diagnosed and are looking for information. The owners and moderators of the group also are very diverse in the years their disorder(s) and their experience levels. They have a combined 65+ years that their condition(s) have affected their lives. By no means are they experts or doctors but have been through a lot of what some of the members are just starting to go through.

The group also has doctors that are world renowned experts in the chiari and other related disorders answer a variety of questions each month and also "weigh in" with their opinions.

We also have two other groups. Chiari Kids and Chiari Parents If you would like to join our support group(s) Click Here to go to the "Support/Information Group" section.

Also, please check out the "Regional Support/Information" groups section. Click Here to go to that section.

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Where Can I Find A Doctor For Chiari?

CCI has compiled a map and list of doctors that have been recommenced by members of our support group.

Chiari and Related Disorders Doctors

**Please read the disclaimer that is located on the doctors page

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How Many People Does Chiari Affect?

There are no "true" numbers on how many people have Chiari. However, it is estimated that anywhere from 200,000 to 2,000,000 people are affected in the United States. It is estimated that 1 in 1,000 are have chiari symptoms.

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What are some disability hints?

Most people with chronic problems like Chiari have some kind of depression. This is an important aspect of disability. If you have experienced any depression or other related problems make sure that they are listed and explained during the application process. Here are some additional tips:

Keep a journal of all of your doctor visits and the way you are feeling each day
Take your time when you fill out the forms and make sure you explain everything in detail
Try and get as many doctors on your side as possible. They will ask the doctors their opinion on disability. So, explain to every doctor what is going on and why you cannot work no matter what doctor it is.
Each time you go to a doctor or facility make sure you tell them ALL of your conditions, these will be documented and given to SS as part of their decision process
Do not hesitate to go to the doctor if something different happens or you are not comfortable with the way you are feeling. (This is a good general rule)
Once you apply call SS often and check status. Once it is in the department of determination tell them EVERY TIME you go to a doctor, ER, or get admitted to the hospital. This is very important because once they pull your records from the doctors or facilities they will not pull them again unless you tell them

Social Security uses a Physical Residual Functional Capacity Assessment. Click Here for a copy of the form.

Social Security also has a Residual Functional Capacity Assessment for Chiari/Syringomyelia. Click Here for a copy of the form.

These form may be helpful in the process of getting disability.

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What are some insurance hints?

Let me start by saying some doctors are going "non par" because insurance companies pay what is called "reasonable and customary fees". A surgeon could charge $10,000 and insurance will come back and say charge what you want, but we are only going to pay $5,000. It doesn't matter if the surgeon is right out of med school, or have years of research to their name. When a surgeon agrees to participate with an insurance company they agree to accept their payment schedule.

When a doctor says they do not accept insurance, that doesn't mean they won't take payment from your insurance company as complete payment, it means they will balance bill. What insurance doesn't pay will be your responsibility

I know that you want to get to the specialist ASAP, but it is best to have your claim approved up front. Know your insurance. If you decide to go ahead with the evaluation and surgery, find out costs before hand. Contact your insurance company to find out what your out of pocket costs will be. Don't be shocked when you are recovering and receive your first bill. Recovery time should be as stress free as possible.

It is much easier to win an appeal before you have had the service. When going to a non- participating doctor, start the ball rolling before your first appointment, not after surgery.

All referrals start with your primary care provider (PCP). Try to find a pcp that agrees you need to be seen by a specialist. It helps to also have a neurologist and even neurosurgeon that will write on your behalf. In my case, my local neurosurgeon didn't have any further answers and he was the one that referred me to begin with.

You only need your pcp's referral to get the ball rolling. Have them send in the pre authorization, and chances are you will be denied. That is when you obtain letters from everyone else. Send your own letter, along with papers written by the surgeon or doctor you want to see. Get your Human Resource department involved as well. Have them write letters supporting the need to go out of network.

Most insurance companies have Case Managers that work with the patient. Your pcp has to submit a request for a case manager. My case manager has been helpful when it comes to explaining what all that legal talk is about, and she has also told me what to write in an appeal letter. Ask for a case manager to help facilitate matters.

After you have sent your letter, call your insurance agent daily. Ask who has your claim, and ask if there is anything else they need to make the decision. Ask to speak to the person that is evaluating your claim. (chances are they won't let you talk to them, but it is worth a try). Keep a journal stating who you talked to, date, and what was said.

If you are denied again, the next step is to go in front of an insurance board. I had a date, but they approved my claim the day before my meeting. From what I understand the board is made of doctors, they ask all kinds of questions, so educate yourself before hand.

Each state has an Insurance board. Your pcp will know the contact information. If you are having problems with your insurance company you can lodge a complaint with the Insurance board.

Contacting your State Representative is another route to take. They can write letters on you behalf.

When talking to insurance companies, talk money. It will cost less to see a specialist the first time, then to have to go through more testing, doctors appointments, and further surgery if the first surgery wasn't a success.

If you feel you have Ehlers Danlos Syndrome (EDS), or any of the related disorders, bring this up in your letters as well. If surgery isn't done correctly there will be further complications. EDS is a new discovery, if during surgery too much bone is removed you will most likely wind up with cervical instability. Here are a couple of links so you can educate yourself about EDS.

http://www.ednf.org/
http://www.ehlersdanlos.ca/

The same goes for anything else you have related to Chiari such as, syringomyelia, hydrocephalus, tethered cord, basilar impression, cervical instability, retroflexed odontoid, you get the drift.

State that seeing a neurosurgeon that specializes in Chiari will lower the cost. Explain that several other issues may complicate treatment and the need for further surgery is high unless you have proper treatment the first time around.

It is possible to have your office visit and surgery paid for except for your normal co-pay, it just takes a little hard work.

Here are a few online links that will also help with appeals, there are many links on the web, just do a search on medical insurance appeals:

http://www.healthsymphony.com/appealing.htm
http://insurance.lawyers.com/Appealing-Health-Insurance-Denials.html
http://www.healthinsuranceinfo.net

Additional information provided by Amanda Baker:

When you have two insurances, you're required to make them known to each other so they can work out between them who pays what. This is known as "Coordination of Benefits". When you have two insurances, they're managed as either "Coordination of Benefits, or Integrated".

As an example, let's say a facility charges 100k for a surgery...but usual/customary is considered to be 70k by the primary insurance carrier. Let's assume that the primary insurance pays 70% out of network and the secondary pays 60% out of network. With COB, the secondary insurance is not concerned with what/how the primary insurance paid. For example, COB scenario:

1. Primary insurance says UC is only $70000
2. Primary insurance pays 70% of UC ($49000)
3. Remaining balance of $51000 is billed to secondary
4. Secondary insurance considers UC to be $50000
5. Secondary insurance pays 60% of UC ($35000)
6. Patient responsible for remaining $16000

HOWEVER, with Integrated benefits, the secondary will only pay the remaining amount of UC that the primary did not cover. For example:

1. Primary insurance says UC is only $70000
2. Primary insurance pays 70% of UC ($49000)
3. Remaining balance of $51000 is billed to secondary. 4. Secondary insurance doesn't care about the $51000. The patient is responsible for that. They're only concerned with paying the portion of UC that was not covered. The primary insurance ruled that UC was $70000 and paid $49000. SO, there is $21000 left to be paid
5. Secondary insurance pays 60% of remaining UC ($12600)
6. Now, a payment of $49000 and $12600 has been made. This leaves the patient with a total of $61600
As you can see it is better to have Coordination of Benefits. Make sure that you tell every facility you have tests at, and every doctor you see that you have two insurance companies so they are billed appropriately

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Craniocervical fusions, cord tethering, and muscle spasm information

First, some definitions, then some news.

Craniocervical fusion (CCF): a surgery stabilizing the joint between neck and skull, for patients affected by craniocervical instability (CCI), a frequent complication of Ehlers-Danlos Syndrome (EDS) and other connective tissue disorders

CCI: a condition in which the head becomes "wobbly" on top of the neck

EDS: connective tissue disorder, with loose joints, and stretchable Skin

Tethered Cord Syndrome (TCS): medical condition in which the spinal cord is stretched downwards, with clinical effects on legs, bladder, bowel, and lower back

Filum Terminale (FT): structure anchoring the inferior pole of the spinal cord to the tailbone

Section of Filum Terminale: operation in which the filum is cut, to alleviate one of the forms of TCS

And now the news, from TCI.

We have discovered that:

- Patients with EDS have frequently CCI and TCS; in retrospect it makes sense, since the CCI affects the joints and the FT is made of connective tissue; therefore, patients who had a CCF at TCI in the past should get in touch with us to be screened for TCS; some of their persistent or new symptoms could be caused by the TCS

- Since the mid-90's, TCI was the first center to screen TCS in pts with CMI; until 2005, the screening process was performed using the traditional tools; on the grounds of the recent clinical finding about Occult TCS (which was systematically missed with the traditional diagnostic tools), we have modified our screening process, which is now more sensitive and less prone to "false negatives" (= less people with TCS are missed by the screening)

- CCF involves an extensive surgical dissection of the craniocervical musculature; the postop recovery has been traditionally very painful; we are now infiltrating the muscles with Botox at the very end of the surgery; the results have been so far very encouraging; hopefully, the pain stories due to postop muscle spasm will become an obsolete item

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Why are there different outcomes of chiari surgeries? Some seem to be more luck than others?

For two main reasons:

* The surgery is the right one for that patient, but it is flawed by complications
* Or the surgery has no complications, but is the wrong one for that specific patient

The list of the complications is long:

* CSF leak
* Meningocele (= the dural patch inflates like a sail)
* Pseudomeningocele (= CSF leaks out of the dural patch and pools in the soft tissues)
* Cervical instability (in case of extensive cervical laminectomies)
* Bacterial meningitis
* Aseptic meningitis (it is not an infection, but an inflammatory reaction)
* Soft tissue infection (i.e. MRSA)
* Excessive scar retraction
* Intracerebellar Hemorrhage
* Stroke (PICA and vertebral)
* Injury of brainstem or cranial nerves
* Etc

Sometimes there are no complications, but the surgery which was performed was the wrong one for that specific patient.

Examples:

* If a Chiari patient has also a severe brainstem kinking by a retroflex odontoid, the posterior decompression will aggravate such condition
* If a patient has an acquired Chiari secondary to hydrocephalus, or a mass in the posterior fossa, the decompression will not address the main problem, and the symptoms will recur
* If a Chiari patient has an underlying upper cervical instability, a C1 laminectomy will aggravate this condition
* If a Chiari patient has an undiagnosed tethered cord, the standard Chiari decompression will not address the main pathology
* And more like that

Any Neurosurgeon can open a book and learn about the standard operation. Aneurysm surgery is definitively more technically complicated than a Chiari decompression, and any neurosurgeon can clip an aneurysm. You draw the conclusion.

The Chiari experts, on the other hand, can:

* Minimize the incidence of complications (remember: nobody is perfect)
* Solve or "patch" the complications when they occur
* "Smell" if there is something else cooking aside from the Chiari and adapt the surgical plan to the given situation

Take home messages:

* Even in the best of hands, complications do happen
* In Chiari, there is no "one-size-fits-all" surgery

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Medical and Educational Recommendations For Children Diagnosed with CM I

Chiari I Malformation is a congenital malformation of the skull characterized by a small posterior cranial fossa and impaired spinal fluid flow. The result is a series of chronic and often debilitating symptoms including, but not limited to: headaches, ocular symptoms, otoneurologic symptoms such as vertigo and imbalance, cranial nerve and brain stem disturbances, bowel and or bladder dysfunction and other nonspecific systemic symptoms. This disease and its complications are characterized by remissions and exacerbations, even after surgical repair. This condition is life long and can affect every aspect of one's life.

We request that you provide the student with accommodations based on Section 504 of the Rehabilitation Act of 1973:

http://www.wrightslaw.com/info/sec504.index.htm

Individuals with Disabilities Act (IDEA 2004):

http://www.wrightslaw.com/idea/index.html

1. If possible the child should be placed in a small class, if that's not possible then a para-professional may be needed to ensure that they are monitored and assessed for proper body positioning, maintain neutral neck position during all activities, and monitor for signs and symptoms of headache, neck pain and/or numbness or tingling in the hands.

2. If the child moves between classes, if possible, classes should be grouped together to minimize excessive walking between classrooms which would increase fatigue.

3. The child should be given extra time for test taking and if necessary provided a test reader and/or note taker. Patients may have difficulty with their daily functioning because of headache and/or vision problems.

4. The child should be provided with a second set of text books to prevent carrying books back and forth between home and school for homework.

5. The child should undergo additional psycho-educational testing to see if they may need other academic services, as well as possible physical and occupational therapy services.

6. Adaptive Physical Education - the student should not participate in any activities that may produce a whiplash-like injury, e.g. dodge ball, other contact sports, trampolines, roller coasters, bumper cars, horseback riding or climbing over 5 feet. Swimming is allowed, but not diving. Weight training and/or strengthening is permissible, but weights must be kept to a maximum of 10 to 15 pounds.

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Information about supplements and herbal medicines

Supplements and herbal medicines have been around since the dawn of time. They do have therapeutic effects, can be beneficial as part of a therapy, and are the object of a multimillion dollar business.

The problem with their use is represented by their interaction with prescription drugs,anesthesia, and intraoperative hemostasis.

Many of these alternative drugs come from small companies, with suboptimal quality control. The dosage, the concentration, the bio-availability (= how much drug actually reaches the target out of a give dose), and the purity of the drug are often lacking precision. The purity issue is an important one, since traces of other drugs processed at the "mom-and-pop" plant can contaminate the batch, with results you can imagine.

These "imperfect" drugs magnify the problem of drug interaction. When more than on drug is taken, they interfere in each other's function; these interactions are documented and predictable for prescription drugs. Whenever a herbal medicine is involved, the interactions are less predictable.

Some of the herbal medicines interfere with intraoperative hemostasis, and can give the surgeon a hard time in controlling blood losses.

Bottom line:
1. Be careful
2. Be aware
3. Inform your doctor about them
4. Inform the presurgical testing personnel about them

Paolo Bolognese, MD

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Hospital "Survival Bag"

1. We bought a new pillow and sewed a seam down the center of it; this divided the contents in two. We covered it with a soft pillowcase. (use a colored pillowcase, so the pillow can be easily identified.) My daughter used this the whole time in the hospital and even after we were home. Having a pillow like this helps to take the pressure off the incision.

2. A soft top sheet instead of the hospitals sheets. We have ones that are like t-shirt material and colorful for easy identification, so it doesn't get lost in their laundry.

3. A few favorite stuffed animals to take into surgery. Even though my daughter was 16, she had 3 favorite ones that she took in with her. We made ID bands and placed on the stuffed animals so they wouldn't get lost. The surgery staff made sure we got them back after her surgery started.

4. We brought some food that we knew our daughter would eat. She always eats certain things when she doesn't feel well, so we made sure we had those along.

5. Bring tops that either button up the front or zip. We brought tank tops with spaghetti straps and my daughter had a hard time getting them on and off with the incision and with the IVs. She wore comfortable pajama pants to the hospital, during the hospital stay and on the way home. Sometimes she wore long ones and sometimes she wore shorts. It was much more comfortable than a hospital gown.

6. Snacks for the parents, because at times you may not want to leave the room.

7. CD player and headphones. My daughter used this all the time. It drowns out the hospital noise and helped her to relax and sleep.

8. Calling card. When she felt up to it, we called her friends and let her talk for a few minutes.

9. We had at least 6 pillows in the car for the ride home. My daughter was given a pain pill before she left and she had another something along the way. Unfortunately we left during rush hour traffic and what should have been a 2 hour ride was 4 1/2.

10. Definitely lotion for patient and parents. The air is very dry in the hospital and the soap is harsh and your hands/skin can get really dry.

11. Another suggestion is a pen and notebook. Every day we wrote down questions for the neurosurgeon. He would answer each and every one and he even took the time to write some of the answers himself as well as verbally tell us.

12. Camera (we took a lot of pictures)

13. A small tape recorder to tape conversations with the doctors

14. A hot/cold pack to use at the hospital when the nurses are taking too long and to use on the plane

15. A book or cross-word puzzle

16. A robe

17. An address book of numbers to look up those who are waiting for that "after surgery" call

18. Ear plugs

19. A sleep mask

20. Mints, lozengers or candy

21. Change for the pop machines

22. Cloraseptic spray or lozengers (if your doctor allows you)

23. High protein snacks like protein bars, nuts, etc.

24. A box of chocolates or candies to let the nurses sneak a piece when they are in the room (helps to make them a little quicker to answer your calls too.

25. Lotion; toothbrush, toothpaste; a comb (some hospitals supply you with these items, some do not, it also makes it cheaper because you can decline the hospitals and not pay $10.00 (an exaggeration) for a tube of toothpaste!

26. Slippers that have tread on the bottoms

27. Cell phone charger

28. A garbage bag for your dirty clothes